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Intensive treatment including surgery for patients with trisomy 13 (T13) remains controversial. This study aimed to evaluate the safety and efficacy of noncardiac surgical intervention for T13 patients. Medical records of patients with karyotypically confirmed T13 treated in the neonatal intensive care unit in Nagano Children's Hospital from January 2000 to October 2016 were retrospectively reviewed, and data from patients who underwent noncardiac surgery were analyzed. Of the 20 patients with T13, 15 (75%) underwent a total of 31 surgical procedures comprising 15 types, including tracheostomy in 10 patients and gastrostomy in 4. Operative time, anesthesia time, and amount of bleeding are described for the first time in a group of children with T13. All the procedures were completed safely with no anesthetic complications or surgery-related death. The overall rate of postoperative complications was 19.3%. Patients receiving tracheostomy had stable or improved respiratory condition. Six of them were discharged home and were alive at the time of this study. These results suggest at least short-term safety and efficacy of major noncardiac surgical procedures, and long-term efficacy of tracheostomy on survival or respiratory stabilization for home medical care of children with T13. Noncardiac surgical intervention is a reasonable choice for patients with T13.

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IntroductionThe mRNA‐based vaccine was released as a COVID‐19 prophylactic; however, its efficacy in organ transplant recipients is unknown. This study aimed to clarify this in liver transplant recipients.MethodsHerein, liver transplant recipients from two hospitals who received vaccines were included. Immunoglobulin‐G antibodies against the spike and nucleocapsid proteins were measured chronologically after the second, third, and fourth vaccine doses.ResultsAntibody levels in 125 liver transplant recipients and 20 healthy volunteers were analyzed. The median age at transplant was 35 (interquartile range 1, 53) years, and the period between transplant and the first dose was 15.2 ± 7.7 years. After the second and third doses, 89.1% and 100% of recipients displayed a positive humoral response, respectively. Anti‐spike antibodies after the second dose were significantly reduced at 3 and 6 months, compared to that at 1 month (26.0 [5.4, 59.5], 14.7 [6.5, 31.4] vs. 59.7 [18.3, 164.0] AU/mL, respectively, p < 0.0001). However, a booster vaccine significantly elevated anti‐spike antibodies in LT recipients (p < 0.0001) as well as in healthy controls (p < 0.0001). Additionally, the decay rate was comparable between the transplant recipients and controls (2.1 [0.8, 4.5] vs. 2.7 [1.1, 4.1] AU/mL/day, p = 0.9359). Only 4.0% of vaccinated transplant recipients were positive for anti‐nucleocapsid antibodies.ConclusionLiver transplant recipients can acquire immunity similar to that of healthy people through vaccination against SARS‐CoV‐2. The antibody decay rate is the same, and booster vaccinations should be administered similarly to that in healthy individuals.

Extraosseous ewing sarcoma (EES) is a rare soft-tissue tumor usually found in the extremities or paraspinal region. We describe the case of a 4-year-old boy with a large cystic mass in the mesentery diagnosed as mesenteric lymphangioma preoperatively and as EES after partial resection and histopathological examination. EES in the mesentery is extremely rare, with only 2 reports described in the English literature. This represents the first report of EES in a child.

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Background: Children who require liver transplantation (LTx) for end-stage liver disease generally have severe growth retardation. After LTx, recipients experience catch-up growth (CUG), though there are not a few cases resulting in short adult height. The aim of the study was to determine decades-long CUG trends and risk factors for short adult height after pediatric LTx. Methods: We examined long-term trends of height Z-score (normalized with the mean of the values is 0 and the standard deviation is 1 using age-and sex-specific references for the general population) in a single-center retrospective cohort of 117 pediatric LTx recipients survived >5 years. The risk factor analysis for short adult height were performed on 75 patients who reached adult height. Results:The median age at LTx was 1.3 years and most primary diagnoses were biliary atresia (77%). Mean height Z-score of pre-LTx and 1, 2, 5 and 8 years after LTx were -2.26, -1.59, -0.91 and -0.59, respectively. After that point, the data plateaued until 20 years. Mean final adult height Z-score was -0.87. In the multivariate analysis, older age at LTx (odds ratio [OR], 1.22 by 1 year; 95% confidence interval [CI], 1.06-1.40; P=0.002), lower height Z-score at LTx (OR, 0.46 by 1 point; 95% CI, 0.29-0.71; P<0.001) and post-LTx hospital stay 60 days (OR, 4.95; 95% CI, P=0.015) were identified as independent risk factors for short adult height. Conclusions: Marvelous CUG was observed after LTx, nevertheless the final adult height was inadequate. For healthy physical growth, LTx should be performed as young as possible and without severe growth retardation, and if growth is inadequate after LTx, use of recombinant human growth hormone might need to achieve proper adult height.

Background. Although chronic kidney disease (CKD) after liver transplantation (LTx) is a common complication in adults, its long-term significance after pediatric LTx remains unclear. We examined the decades-long transition of renal function and revealed the risk factors for late-onset CKD after pediatric LTx in a single-center retrospective cohort of 117 pediatric LTx recipients who survived >5 y. Methods. The estimated glomerular filtration rate (eGFR) and CKD stages were calculated using serum creatinine. Risk factor analysis for late-onset CKD was performed in 41 patients whose eGFR could be evaluated at >20 y after LTx. Results. The median age at LTx was 1.3 y, and most primary diagnoses were biliary atresia (77%). The mean pre-LTx and 1, 5, 10, 20, and >20 y post-LTx eGFRs were 180, 135, 131, 121, 106, and 95 mL/min/1.73 m2, respectively, with a median renal follow-up period of 15 y. The eGFR declined by 47% at >20 y after LTx (P < 0.001). CKD was observed in 8%, 19%, and 39% of cases at 10, 20, and >20 y after LTx, respectively. In patients receiving cyclosporine, trough levels were 1.5 times higher in those with CKD up to 10 y after LTx. The multivariate analysis showed that older age at LTx (odds ratio, 1.3 by 1 y; P = 0.008) and episodes of repeated/refractory rejection (odds ratio, 16.2; P = 0.002) were independent risk factors of CKD >20 y after LTx. Conclusions. In conclusion, renal function deteriorates slowly yet steadily after pediatric LTx. Long-term careful surveillance is essential after pediatric LTx, especially in repeated/refractory rejection or long-term high trough-level use of cyclosporine cases.